#Pediatric Laryngomalacia

[drdushyantmandlik]

Pediatric Head and Neck Conditions: Understanding Treatment Approaches for Children

Pediatric head and neck conditions encompass a broad spectrum of disorders affecting children, ranging from congenital anomalies to acquired diseases. Understanding treatment approaches for these conditions requires a multidisciplinary approach involving paediatricians, otolaryngologists (ENT specialists), pediatric surgeons, radiologists, and other specialists. Here's an overview of some common pediatric head and neck conditions and their treatment approaches:

Cleft Lip and Palate: Cleft lip and palate are among the most common congenital anomalies affecting the head and neck region. Treatment typically involves surgical repair, which may be performed in stages starting in infancy and continuing into childhood.

Lymphadenopathy: Enlarged lymph nodes in the head and neck region can result from infections, inflammatory conditions, or malignancies. Treatment depends on the underlying cause, which may include antibiotics for infections or further evaluation and management for malignancies.

Thyroid Disorders: Thyroid Cancer Treatment, along with conditions like congenital hypothyroidism and thyroid nodules, can impact children. Treatment may entail hormone replacement therapy to manage hypothyroidism or surgical intervention to remove cancerous nodules when necessary.

Otitis Media: Middle ear infections are common in children and may require antibiotic treatment. In some cases, particularly if infections are recurrent or severe, ENT evaluation for possible placement of tympanostomy tubes may be necessary.

Sinusitis: Sinus infections can occur in children, often as a complication of upper respiratory tract infections. Treatment may involve antibiotics, nasal saline irrigation, and supportive care to alleviate symptoms.

Pediatric Neck Masses: Neck masses in children may be due to a variety of causes, including benign or malignant tumours, cysts, or inflammatory conditions. Treatment depends on the underlying aetiology and may involve observation, surgical excision, or other interventions.

Hearing Loss: Various causes of hearing loss in children exist, including congenital anomalies, infections, and acquired conditions. Treatment may involve hearing aids, cochlear implants, speech therapy, or surgical correction depending on the cause and severity of hearing loss.

Airway Disorders: Conditions such as laryngomalacia, tracheomalacia, or subglottic stenosis can affect the pediatric airway. Treatment may involve observation, medical management, or surgical interventions such as airway reconstruction.

Dental and Oral Health: Dental caries, malocclusion, and other oral health issues are common in children and may require interventions ranging from preventive measures such as fluoride treatments and sealants to orthodontic treatment or surgical correction.

Trauma: Head and neck trauma in children may result from accidents, falls, or non-accidental injuries. Treatment varies depending on the nature and severity of the injury but may involve stabilization, surgical intervention, and rehabilitation.

When managing pediatric head and neck conditions, particularly concerning Head and Neck Cancer Treatment in Ahmedabad, it's imperative to recognize the distinctive anatomical and physiological variances in children compared to adults. Children's smaller airways, developing immune systems, and the potential impact of treatment on growth and development necessitate tailored approaches.

[earnoseandthroatdoctors]

Pediatric Laryngomalacia - Coastal Ear Nose & Throat

Laryngomalacia is the most common cause of raspy, noisy breathing in infancy. It happens when the structure of the larynx is floppy and malformed. This causes the tissues to fall over the airway, partially blocking it and creating noise when breathing.

[crimson-rehabilitation]

Rare Case Primary Report: Xia Gibb’s Syndrome_Crimson Publishers

Abstract

Xia-Gibbs syndrome (Mental retardation, autosomal dominant 25; MRD25) (MIM 615829), first described in 2014, is a rare autosomal dominant disease characterized by mental retardation, developmental delay, speech delay, structural brain anomalies, hypotonicity, protuberant eyes, visual problems, laryngomalacia and low sleep quality. It occurs due to heterozygous mutations in the AT-hook DNA binding motif containing 1 (AHDC1) gene on chromosome 1p36. Research reports published in 2020 by Ping et al. [1], states that about 100 patients worldwide have been identified, all of them in the pediatric age or adulthood. To date, no fetal XGS has been reported [1-4]. Xia-Gibbs syndrome (XGS) is a recently described neurodevelopmental disorder due to heterozygous loss-of-function AHDC1 mutations. XGS is characterized by global developmental delay, intellectual disability, hypotonia, and sleep abnormalities. We are here to report our primary observations and findings of a diagnosed case of Xia Gibb’s Syndrome referred for Physiotherapy with signs of development delay, atonia, joint laxity and related features.

Read more about this article: https://crimsonpublishers.com/epmr/fulltext/EPMR.000562.php

Read more Crimson Publishers Google Scholar  Articles:  https://scholar.google.com/citations?view_op=view_citation&hl=en&user=MAr8S6kAAAAJ&citation_for_view=MAr8S6kAAAAJ:M3ejUd6NZC8C

[crimsonpublishers-pediatrics]

Index of Suspicion: Colonic Extravasation of Intravenous Contrast After Cardiac Catheterization _ Crimson Publishers

Index of Suspicion: Colonic Extravasation of Intravenous Contrast After Cardiac Catheterization by Sukumar Suguna Narasimhulu in Research in Pediatrics & Neonatology

We present a 4 month old black male infant with prenatally diagnosed complex congenital heart disease with single ventricle physiology and normal amniocentesis. He was born prematurely at 35 3/7 weeks with a birth weight of 3.2kg to a 33 YO, G6P4 female via C-section for Breech presentation and non-reassuring fetal heart tracing. Pregnancy was also complicated by non-compliant insulin dependent diabetes mellitus. APGAR scores at birth were 1, 4 and 8 at 1, 5 and 10 minutes respectively, with the patient requiring resuscitation with intubation and mechanical ventilation. He was admitted in the cardiac ICU for further management.

His cardiac diagnosis was right ventricular dominant complete atrioventricular canal defect, in the setting of double outlet right ventricle with severe pulmonary stenosis. His physical exam was positive for stridor secondary to severe laryngomalacia, ankyloglossia, micropenis, anal stenosis with an abnormal anal verge and posteriorly placed anus.

https://crimsonpublishers.com/rpn/fulltext/RPN.000549.php

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[phgq]

Child with rare disease fights for his life

#PHnews: Child with rare disease fights for his life

MANILA – At first glance, one-year-old Dimitri Stefan Harper Manalaysay looks like any other toddler who lives a normal life.

But Dimitri is different. 

The bright-eyed boy who makes everyone happy with his infectious smile was born with Rubinstein-Taybi syndrome, a rare condition that affects one in 125,000 newborns.

Rubinstein-Taybi syndrome is a rare genetic disorder that may affect many organ systems of the body, according to the definition provided by the National Organization for Rare Disorders (NORD) on its official website.

Babies with Rubinstein-Taybi syndrome usually have distinctive facial features, growth delays, intellectual disability, characteristic abnormalities of the head and face (craniofacial dysmorphism), breathing and feeding difficulties, and urogenital difficulties (dysphagia).

Dimitri has spent only four months at home, staying most of the time at the hospital – two weeks at the neonatal intensive care unit (ICU) and seven months at the pediatric ICU (PICU), his mother, Dea, told the Philippine News Agency (PNA).

‘Happy, normal baby’

If you look at Dimitri, you would think of him as a “happy” and “normal” baby, Dea said.

Dimitri, she said, even appears not to have an intellectual disability as he is jolly and loves the interaction with his family.

“Happy baby po siya. Sobra. Napakadaldal. Napakaharot. Ang lambing. Hindi mo po iisipin na meron siyang syndrome kasi sinasabi nila, kapag meron kang Rubinstein-Taybi, syndrome, intellectually, wala kasi ‘di ba po severe mental disability siya, sabi nila. Pero si baby, nagre-respond siya (He is a happy baby. He really is. He talks a lot. He is very jolly. He is sweet. You would not think he has Rubinstein-Taybi syndrome because those who are suffering from the rare disease have mental disabilities. But our baby is responsive),” Dea said.

HAPPY BABY. Despite his condition, Dimitry managed to smile during his first birthday celebration on Jan. 5 (Photo courtesy of Hyron Manalaysay)

Dimitri also likes hearing his name, as well as the words “I love you,” she said, adding that her son also loves listening to songs from Cocomelon, an American Youtube channel that features various songs for children.

“Kapag narinig niya ‘yung intro ng Cocomelon, ‘yung face niya talaga, magla-light up. Tapos kapag sinabihan mo siya ng ‘I love you,’ tatawa siya. Kapag tinawag mo ‘yung pangalan, magre-respond siya. As in parang normal baby (When he hears the intro of Cocomelon, his face lights up. When you tell him ‘I love you,’ he smiles. When you call his name, he responds. He acts like a normal baby),” Dea said.

On top of Rubinstein-Taybi syndrome, Dimitri is also battling multiple sepsis, hypoglycemia, pneumonia, laryngomalacia, bronchiectasis, congenital glaucoma, and gastroesophageal reflux disease.

His doctors said he might also be suffering from Hirschsprung disease, a birth defect that prevents newborns and young children from passing stool because some of their intestinal nerve cells do not develop.

“‘Yung Hirschsprung disease daw po, partner talaga ni Rubinstein. Hindi pa raw po kasi confirmed ‘yung Hirschsprung niya (Supposedly, Hirschsprung disease is a partner of Rubinstein. However, they could not confirm yet whether he has Hirschsprung disease),” Dea said.

GOD'S MIRACLE. Dimitri's mother, Dea, considers him as "God's miracle" as he is able to withstand all the medical procedures that he had to go through. (Photo courtesy of Hyron Manalaysay)

The ordeal Dimitri’s family has to go through is no joke, especially now that he is in a “very critical” condition.

After four months of home care, he was rushed to the hospital on January 10, just five days after he celebrated his first birthday, due to high-grade fever and sudden bloating of his tummy.

His parents, Dea and Hyron, were not expecting that Dimitri would be brought back to the hospital, as their child even managed to play with them that day.

Dimitri, who is fed through a gastrostomy tube since he was just three months old, also underwent a colostomy during his recent stay at the hospital because part of his small intestine had a small hole.

He was once again brought to the PICU, fighting for his life after being declared under “very critical” condition.

Dea said doctors had told them to prepare themselves, in case the boy could no longer bear the pain he has long been enduring.

“Right now, very critical siya. Sinabihan na kami ng doctor na mag-ready kami kasi daw anytime… Ganoon na siya kalala (Right now, he is in very critical condition. We’ve been told to get ready anytime… His situation continues to worsen),” she said.

Dea said Dimitri’s doctors are already losing hope but the baby is still fighting.

She said her son was even responding when she visited him at PICU on Friday.

“Fighting pa rin po siya. Pumayag po sila na pumasok ako kagabi kahit 15 minutes. Nakausap ko po si baby. Nagre-respond po siya (He’s still fighting. They allowed me to check on him for 15 minutes. I was able to talk to him. He was responding),” Dea said.

She even saw Dimitri’s eyes move when she told him “I love you.

“Responding po siya pero hindi siya nagigising. Kumbaga, nagkakaroon lang po siya ng reaction sa ginagawa ko. Halimbawa po, hinawakan ko yung kamay niya, nakakahawak siya pero hindi sobrang higpit katulad ng ginagawa niya sa bahay. Tapos kapag kinakausap ko siya, pag sasabihan ko siya ng ‘I love you,’ hindi siya nagre-respond pero nakikita ko yung mata niya, gumagalaw (He’s not awake. There’s just a reaction in everything I do. I held his hand, and he held mine as well. I talked to him and told him, ‘I love you.’ He was not responding but his eyes moved),” Dea said.

10K tote bags

TOTE BAGS FOR SALE. Dimitri's parents are targeting to sell around 10,000 canvas tote bags for his medical expenses. (Photo courtesy of Hyron Manalaysay)

They need to sell some 10,000 tote bags for Dimitri so he could undergo laparoscopy and other surgical procedures, once his condition becomes stable.

“‘Yung goal talaga naman is makabenta kami ng at least 10,000 pieces. Alam ko very ambitious pero umaasa kami na baka sakali (Our goal really is to sell at least 10,000 pieces. I know it’s very ambitious but we are hoping to achieve our goal),” Dea said.

As of now, only 260 tote bags have been sold, she said.

“Kailangan pa rin po namin (ng pera for Dimitri’s medical expenses) kasi ‘yung doctor na po niya dito ang nagsabi na magtatagal pa rin siya kung halimbawa daw po, ma-survive niya ang episode ng septic shock niya. Kapag nawala na lahat ng infection sa katawan niya, mag-proceed na sa surgery (We still need money for Dimitri’s medical expenses because his doctors said he would stay longer in the hospital if he survives the episode of septic shock. When he is able to fight the infection, the surgery would proceed),” Dea said.

Each tote bag costs PHP300.

Those interested can choose varieties of designs printed on each bag.

The proceeds will be spent on the operations that would be performed on Dimitri.

Anyone who wants to help Dimitri can reach Dea and Hyron through mobile phone numbers 0998-366-1453 and 0949-303-3447. (PNA)

***

References:

* Philippine News Agency. "Child with rare disease fights for his life." Philippine News Agency. https://www.pna.gov.ph/articles/1130556 (accessed February 13, 2021 at 11:19PM UTC+14).

* Philippine News Agency. "Child with rare disease fights for his life." Archive Today. https://archive.ph/?run=1&url=https://www.pna.gov.ph/articles/1130556 (archived).

[larynx-lover]

Dehra we all know that you want ENT but there are two things I doubt about ENT 1- when I asked my friends about ENT they replied with one word (tonsils) because based on my classmates that's all what the ENT doctor do 2- what if you didn't get you ENT residency is there a second specialty that you are interested in?

Oh boy! Here we go, everyone!

1) ENT doctors have the ability to do SO much beyond tonsils. I will say that tonsils, adenoids, and tubes are kind of the “Bread and butter” of general otolaryngology. Ear infections are the #1 reason why parents bring their kids to the doctor. Even a lot of adults get tubes for ear infections. Tonsillitis is super annoying, and enlarged tonsils can also play a role in sleep apnea. But there is so much more to ENT than just those things. And how much a general otolaryngologist practices these things is really up to the individual doctor and their training! For example:

Head and Neck Surgery: Large cancer resections of the head and neck, composite resections, parotidectomies, thyroidectomies, parathyroidectomies, laryngectomies, neck dissections, wide excisions, etc…

Reconstruction/Microvascular surgery: A lot of ENT surgeons do their own reconstructions after they resect cancer! Lat flaps, pec flaps, radial free flaps, anterolateral thigh free flaps, ORIF of mandible/maxilla, trauma reconstructions

Otology: Hearing loss, temporal bone resections, mastoidectomies, cholesteatomas, joint cases w/ neurosurgery to get access to the brain, tympanoplasty, cartilage grafts, sphenoid/temporal fractures

Laryngology: node/polyp removal, airway dilation, esophageal dilation, laryngeal cancer resection, laser ablation of the vocal cords, vocal cord contouring, micro flaps, medializations w/ temporary filler or permanent thyroplasties, laryngomalacia, Werner’s, 

Rhinology: Endoscopic sinus surgery, rhinoplasties, polyp removal, turbinate reductions (tbh this is the field I know the least about)

Facial plastics: Rhinoplasties, face lifts, reconstruction, flaps, fat grafts… pretty much what it sounds like

There are also allergy and sleep specialists within ENT, not to mention Peds ENT which is still a lot of tubes and tonsils, but also management of the pediatric airway, pulling coins out of tracheas and stuff. And the best thing about ENT is that you can tailor your practice to whatever suits you. Are you a clinic person? OR person? Office procedures? Once you are out of residency you have so much control over your lifestyle. So I hope I’ve cleared some of that up! ENT is a broad field with a lot of diverse procedures, innovative research, and opportunities!

2) Plastic Surgery is the field I like the most besides ENT, but it isn’t really a “back up” you know? It’s just as hard to get into. So I don’t really have a great backup plan. I’m just gonna go for it. All of the faculty I’ve talked to have told me pretty confidently that I’ll be fine.

[juniperpublishersanesthesia]

Successfully Anesthetic Management in a Rare Syndrome, Noonan Syndrome: Case Report-Juniper Publishers

Abstract

Noonan's Syndrome (NS) is a multisystem congenital disorder and characterized by fascial and pysical fatures along with congenital heart disease. In these patients, fascial features include short webbed neck, micrognathia, limited mouth opening and high arched palate can be a big problem for the tracheal intubation. Most commonly associated cardiac anomaly is pulmonary stenosis (>50% of cases). In these patients anesthetic management is important because of difficult airway and severe cardiac abnormalities. Here, we have reported the preoperative evaluation and anaesthetic management of a child with NS complicated by pulmonary stenosis and laringomalasia.

Keywords: Congenital heart surgery; Noonan's syndrome; Difficult intubation; Pulmonary stenosis

Abbreviation: NS: Noonan's Syndrome; PS: Pulmonary Stenosis; HOCM: Hypertrophic Obstructive  Cardiomyopathy; ASD: Atrial Septal Defect; RVOTR: Right Ventricle Outflow Tract Reconstruction; BIS: Bispectral Index; FOB: Fiber-Optic Bronchoscopy; IOC: Index Of Consciousness

Introduction

Noonan's Syndrome (NS) is a multisystem congenital disorder. Pulmonary stenosis (PS) and hypertrophic obstructive cardiomyopathy (HOCM) are the most common cardiac anomalies found in combination with other lesions like atrial septal defect (ASD), ventricular septal defect, tetralogy of Fallot, aortic stenosis, coarctation of aorta, Ebstein's malformation, total anomalous pulmonary venous return, ostium primum ASD and patent ductus arteriosus [1,2]. The potential anesthetic problems presented by a patient with Noonan's syndrome may be due to impairment of cardiopulmonary function, the possibility of a difficult airway [1,2].

In this case report we have described the preoperative evaluation and anaesthetic management of a child with NS complicated by pulmonary (PS) stenosis and laringomalasia. The anesthetic management in these patients should be carried out with careful preoperative evaluation of physical status, to maintain hemodynamic stability and especially the difficult endotracheal intubation should be kept in mind [3]. In this case, we have reported an anesthesic management of a 13 months old girl with Noonan Syndrome underwent congenital heart surgery under general anesthesia suggesting difficult tracheal intubation on preoperative physical examination and evaluation of the anesthesiologic aspects of this sendrome.

Case Report

A thirteen-months-old, 5kg weight girl with clinical diagnosis of NS was admitted to our hospital for the operation of PS. In echocardiography PS had detected at birth. In the third month balloon valvuloplasty was performed. Pulmonary gradient was 68mmHg. Because of growth deficiency RVOTR (Right Ventricle Outflow Tract Reconstruction) was planned for her.

In the preoperative evaluation she had pes plano valgus, wide hemangioma in face, short stature, micrognatia, macroglossia, short steatore. She had gone to operation for PS under general anesthesia. Monitoring was done throughout the operation; vitals were recorded on monitors every 5 minutes. In addition to standart monitoring, sedation level monitored with Bispectral Index (BIS), regional oxygen saturation was monitored with INVOS Cerebral Oximeter, which afford dual-site monitoring with pediatric disposable sensor, invasive right arterial pressure also monitored. In case of intubation and airway management difficulties we prapared equipments, fiberoptic laringoscopy for difficult intubation.

General anesthesia was initiated with 2mcg/kg/min remifentanil infusion. After establishing successful bag mask ventilation Cormack-Lehane score 3 were assessed with direct laryngoscopy (macintosh blade, size 2). After two failed intubation attempts with direct laryngoscopy, fiber-optic bronchoscopy (FOB) was carried out and a successful intubation with a FOB was performed. In the meantime, no desaturation occurred in the patient who was ventilated with the mask. Cuffed endotracheal tube number 4 mm internal diameter was safely placed into the trachea without trouble.

Endotracheal cuff pressure was maintained among 1015cm H2O which was continuously monitored till extubation. After confirming effective endotracheal intubation, sodium thiopental 3-4mg/kg and rocuronium 0.6mg/kg were given. Anesthesia was maintained with isoflurane 50% oxygen/air and continuous infusion of remifentanil (0.01-1mcg/kg/min) until the end of surgical procedures with the aim of keeping index of consciousness (IOC) values within 40-60, heart rate and blood pressure within the 30% range. Depth of anesthesia was monitored with index of consciousness (IOC), Morpheus Medical, Barcelona, Spain) [K]. Cerebral (rSO-C) and somatic (rSO-S) tissue oxygen saturation were monitored and were stable during the operation, did not change compared to the initial values.

Data were continuously updated at two readings per second and average recordings saved at 1 minute intervals (Pediatric SomaSensor, Model SPFB, for children 4-40kg by Somanetics Corporation, Troy, Michigan for the INVOS 5100 Cerebral oximeter). Remifentanil, being an ultra short active opioid, was preferred for slow induction of anesthesia being advantageous for hemodynamic stability in this case. At the end of operation the patient was taken to the CICU; mechanical ventilation was done in SIMV mode. Patient was extubated when her spontaneous breathing was adequate 3 hours later after the operation without any problem.

Discussion

Noonan syndrome (NS) with characteristic facial features is one of the most common non chromosomal syndromes presenting to the cardiac anesthesiologist for the management of various cardiac lesions, predominantly pulmonary stenosis (PS) (80%) and hypertrophic obstructive cardiomyopathy (HOCM) (30%) [4,5]. Anesthetic management poses a multitude of challenges, especially related to the airway management and maintenance of cardiovascular stability.

Successful management of the difficult airway implies recognition, adequate preparation, and, finally, familiarity with at least several of the special techniques which may be used for tracheal intubation of patients with a difficult airway [3]. Noonan syndrome is characterized by fascial and physical features, airway abnormalities along with congenital heart disease. In these patients, fascial features include short webbed neck, micrognathia, limited mouth opening and high arched palate. Pulmonary stenosis and hypertrophic obstructive cardiomyopathy are highly prevalent. The anesthetic management is important because of difficult airway and severe cardiac abnormalities [3-6].

Here, we have reported a thirteen-months old patient with characteristic features of NS; micrognathia, limited mouth opening, high arched palate, short stature, macroglossia who underwent congenital heart surgery without any complication in airway management during the operation.Miscellaneous conditions such as macroglossia, micrognathia, short steatore can congest small infant mediastinum compromising airway [3-6]. Since our patient had the characteristic features macroglossia, micrognathia, laryngomalacia; resuscitation and difficult airway management trolley, FOB was kept by the side. Equipment for tracheostomy was made available. We have used FOB for intubation after two inadequate attempts for intubation with direct laringoscopy (macintosh blade, size 2).

The airway trolley was made ready with all the equipment necessary in case of difficult airway management. Arterial hypoxemia and desaturation can occur very rapidly in pediatric patients in view of the decreased functional residual capacity. Therefore, preoperative, intraoperative, and postoperative pharmacology has to be given an in-depth consideration to avoid any incidence of hypotension and hypoxemia [6-8].

After two failed intubation attempts with direct laryngoscopy, a successful intubation with a fiberoptic bronchoscope was performed. After operation the patient was transferred to CICU. We planned to apply fast track extubation that's why we used ultra-short acting opioid, remifentanil during surgery and CICU.

When early extubation is planned, ensuring adequate postoperative pain management is essential. Endotracheal cuff (ETTc) pressure monitored till extubation between the ranges of 10-15cm H2O. The tracheal tube cuff should ideally seal the airway without compromising mucosal perfusion, cuff pressure should be maintained around 10-15cm H2O in critically ill intubated and mechanically ventilated patients. When ETTc pressure exceeds the capillary perfusion pressure of tracheal mucosa, mucosal blood flow is obstructed and may lead to severe even fatal injury including tracheal pain or stridor [9,10].

Early extubation after congenital heart surgery is becoming popular in selected patients. Fast-track cardiac anesthetic techniques lead to earlier tracheal extubation, shorter ICU stays and significant reductions in cost. Remifentanil, ultra short acting opioid, has been used for infants and children to achieve easy, safe and early extubation after major surgeries [11-14]. Achieving adequate depth of anesthesia during surgical procedures is desirable. IOC can be useful in guiding anesthetic dose to avoid risks of intraoperative recall in surgical patients with high risk of awareness; can improve anesthetic delivery and recovery from anesthesia. We used IOC monitoring both perioperative and postoperative period to determine the depth of anesthesia and to maintain optimal conditions for extubation [15,16].

Successful management of the difficult pediatric airway can be challenging and stressful. Anticipation is often key to success, and it is preferable to err on the side of conservatism. In the unanticipated difficult airway, anesthesia personnel must utilize the conservative, «common sense» approach advocated in the ASA guidelines. Successful management of the difficult airway implies recognition, adequate preparation, and, finally, familiarity with at least several of the special techniques which may be used to intubate the trachea of patients with a difficult airway. For successful airway and anesthetic management in a case of NS, anesthesiologists should have thorough and deep knowledge about the various anatomic anomalies and pathophysiologic considerations to prevent any clinical disaster, especially for an elective surgery.

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[fitriarumsari]

13. Dandy Walker Syndrome

“Kondisinya menurun, saturasi oksigennya malam ini rendah” ucap suster Gita lewat telepon. 

“Pantau terus tanda-tanda vitalnya” balas dokter Edwin. 

Dokter Edwin, Sp.A selaku DPJP (Dokter Penanggung Jawab Pasien) yang kondisinya baru saja sampai rumah, tiba-tiba mendadak harus kembali lagi ke rumah sakit.

Namanya Fajar. Sebut saja Muhammad Fajar Al-Biruni. Dia adalah pasien “spesial” di rumah sakit tempatku bekerja. Semua orang pasti mengenal dia. Bagaimana tidak? Dia sudah sekitar 350 hari bertahan di PICU (Pediatric Intensive Care Unit) dengan kondisi kritis.

Fajar didiagnosa mengidap penyakit langka, Dandy Walker dan Patau syndrome, penyakit idiopatik abnormalitas otak akibat kelainan kromosom 13 yg berlebih. Dokter bilang, ini bukan salah Ibunya. Tidak ada yang tahu penyebab mengapa Fajar terkena syndrome langka ini. Bukan karena makanan atau aktivitas ketika ibunya hamil. Syndrome ini memang terjadi akibat gangguan pembelahan sel mitosis saat janin masih di dalam rahim.

Saat masih dalam kandungan, Ibu Fajar sudah didiagnosa oleh dokter obgyn bahwa anaknya kemungkinan besar akan tumbuh abnormal. Namun, Ibu Fajar tetap terus berdoa mudah-mudahan masih ada harapan dan kesempatan untuk Fajar bisa tumbuh normal seperti anak-anak lainnya. Ketika lahir, Fajar didiagnosa menderita: 1. Congenital Heart Defect of VSD, PDA, ASD (atau istilah awamnya bocor jantung) 2. Aspiration Pneumonia sehingga kesulitan bernapas. 3. Hydronephrosis (pembengkakan nefron ginjal) 4. Laryngomalacia 5. Dandy Walker (semacam syndrome langka abnormalitas cerebellum otak untuk mengatur koordinasi otot)

Untuk bernapas, Fajar membutuhkan ventilator, hingga akhirnya dokter Edwin selaku dokter penanggung jawabnya melakukan tindakan tracheostomy (bedah trakea untuk membuka jalur napas) agar Fajar bisa lebih bebas menghirup udara. Beruntungnya, Fajar memiliki Ibu yang sabar. Ibunya setiap hari mengunjungi, mengajak bicara dan menyayangi Fajar layaknya bayi normal lainnya. Suster-suster PICU tak kalah perhatiannya dengan ibunya.

Hari terakhir Ramadhan kemarin adalah hari ke-358 Fajar berada di PICU. Setiap malam Fajar menangis kencang, mungkin karena terasa kesakitan dan demam tinggi. Hingga suster-suster PICU mengajak bercanda 

“Fajar, malam ini mau ngeronda lagi ya? ”

Namun anehnya, setiap Fajar menangis tanpa suara sambil tersenyum, keesokan harinya selalu ada pasien di ruang PICU yang meninggal. Satu per satu teman-teman bayi Fajar meninggalkan Fajar hingga hari ke-358. Teman-teman Fajar satu persatu silih berganti. Ada yang pulang karena lekas membaik, ada yang bertemu Allah lebih duluan. Kata orang, Fajar begitu karena melihat malaikat maut. Menangis tanpa suara, dengan raut wajah terlihat tersenyum.

Fajar sudah berkali-kali ganti antibiotik. Dari mulai Levofloxacin, Amikacin, Cefepime, sampai golongan Carbapenem yakni Meropenem. Kondisinya tetap kritis, koma. Hingga suatu hari, saat Idul Fitri kemarin, Fajar mencapai titik tertinggi sehatnya menurut dokter. Fajar sudah bisa bernapas tanpa ventilator dan sudah tidak demam lagi. Ibu Fajar sempat memakaikan peci kecil sambil memotret anaknya dan mengunggah foto di instagramnya dengan caption “Selamat idul Fitri”.

Terakhir aku melihat Fajar adalah ketika mengecek trolley emergency di PICU. Fajar tertidur pulas dengan suara lantunan al quran di sebelahnya. Subhanallah. Qadarullah saat malam kedua idul Fitri tiba, kondisi Fajar tiba- tiba menurun. Fajar kembali demam, tanda-tanda vital menurun, penurunan kesadaran dan saturasi.

Innalillahi wa innalillahi rajiun. Saat itu Fajar dipanggil untuk menemui Allah setelah hampir satu tahun bertahan di PICU. Mungkin Fajar lelah, Fajar ingin segera bertemu dengan Allah. Semua orang merasa kehilangan, apalagi ibunya, untuk ukuran anak pertamanya.

Ada banyak “Fajar” lain didunia ini yang sedang atau pernah bertahan hidup. Selayaknya dengan ingat mereka, betapa sehat itu terasa sangat berharga. Banyak fenomena dan pembelajaran dari berbagai pasien untuk bisa dijadikan alasan untuk terus bersyukur. Semoga kita tidak lupa untuk mensyukuri nikmat sehat. Semoga Fajar dan “Fajar” lainnya bisa menjadi tabungan syurga untuk orang tuanya di akhirat kelak.

#30DaysWritingChallenge #Day13 #30DWC